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To Refer or Not To Refer: Evaluation is the Question

Shanna Diaz, DO Adult and Geriatric Sleep University of New Hospital November 11, 2016 Objectives

• Have a basic understanding of different sleep disorders • Know risk factors associated with different sleep disorders • Be aware of common associated with sleep disorders • Understand when a sleep evaluation referral is needed Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Disorders • • Sleep Related Movement Disorders Contributors to Sleep Disorders

• Intrinsic abnormalities • Brain chemicals () of sleep and wake • Circadian rhythm Modulators • Superchiasmatic Nucleus • Regulation • Homeostasis Modulators • Sleep Pressure • Autonomic • Hormone regulation • • Testosterone • Estrogen • centers in brain stem • Messages about when and how to breathe Contributors to Sleep Disorders

• Medical illnesses • • Diminished Breathing Effort • Cardiac • Central • Pulmonary disease • • Hypercarbia • Mellitus • Energy Disease • Fluid Balance • Pain • Hyperarousal • Restlessness • Seizures • Hyperarousal Contributors to Sleep Disorders

• Other primary sleep disorders • • Circadian Rhythm Disorders • Parasomnias • Movement disorders Contributors to Sleep Disorders

• Sedating Medications • Anti- medications • medications • Muscle relaxants • Sleep aids • Medications • • Psychotropic Medications • • Antidepressants • Anti- medications Contributors to Sleep Disorders

• Psychiatric and Behavioral factors • Anxiety • • Obsessive Compulsive Behaviors • Cognitive Problems • Caregiver Interactions Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Obstructive Sleep Apnea

• Repeated pauses in breathing interrupting sleep • At least 10 second duration • Oxygen desaturation • Brain interruption • Associated with many medical and behavioral problems • Risk Factors: • Body Habitus • Facial Features • Muscle Tone Sleep Apnea Cycle

• Physiologic stressors: • Cyclic hypoxemia • 11,911 adults –41% systemic hypertension • Strenuous • Sympathetic activation • Reduced total sleep time

Tkacova, Eur Respir J. 2014

Untreated Sleep Apnea 1868 subjects followed 20 years

Incidence of multiple organ higher in OSA Group: 32% with OSA 68% without OSA

• Hypertension: 79% 25% • Coronary heart disease: 56% 24% • : 6% 4% • : 27% 7.5% • Diabetes: 12% 5.4%

Ci SP, 2011 (Article in Chinese) Signs/Symptoms May Vary With Age

• Children • Pauses in breathing • Secondary • Hyperactivity • Morning • Middle Age • Pauses in breathing • • Excessive daytime sleepiness • BMI > 35, > 16/17 • Older > 60 years • Not feeling well rested • ≥ 3 Obstructive Sleep Apnea Symptom: SNORING

• Children • 10% • Age 30 • 20% men • 5% women • Age 60 • 60% men 20-35% of habitual snorers have • 40% women OSA Physical Findings on Exam Which May Predispose to OSA

BMI > 35 Neck Circumference • > 16 in women • > 17 in men Physical Findings on Exam Which May Predispose to OSA

• Crowded Oropharynx • High arched narrow palate • Low laying palate • Large uvula • Narrow posterior oropharynx • Tonsillar hypertrophy

http://yoursmileyourstyle.com/files/2014/05/What-your-dentist-looks-for-in-diagnosing-sleep-apnea.jpg Physical Findings on Exam Which May Predispose to OSA

• Chronic • Nasal speech • Obligate mouth breather • adenoidal hypertrophy • Mandibular retrognathia • Floppy Eye • AKA Ectropion • 38/45 patients (85%) had OSA • 65% had severe OSA

http://www.imo.es/wp-content/uploads/2011/02/ectropion-antes.jpg STOP-BANG: Quick Screening Tool for OSA

http://firstlighthealthsystem.org/wp-content/uploads/2016/04/Stop-Bang.jpg OSA Summary

Most Common Symptoms: • Snoring • Pauses in Breathing • Excessive Daytime Sleepiness Treat to Reduce Morbidities: • Cardiovascular • Neurological • Behavioral Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Biologic Clocks Influence All Circadian Rhythm Disorders

• Misalignment of internal sleep/wake rhythm and the desired (or required) time for sleep

• Desire for sleep and at inappropriate times

• Risk Factors

• Intrinsic brain abnormalities • Blindness • Genetic predisposition Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Insomnia

• Hard to sleep • Difficulty going to sleep • Difficulty staying asleep • Waking up too early • Poor quality sleep • Risk Factors: • Hyperarousal • Environment • Habit •

Acute Insomnia

• Acute Insomnia • < 3 month duration • In isolation • Often a known stressor/cause • Comorbid to medical or condition • Improves with treatment for underlying cause Chronic Insomnia

• Chronic Insomnia • > 3 months • 3+ nights per week • Subtypes: • Psychophysiological Insomnia • “Trained” by habit • Worse in usual sleep environment, better in different environment • Excessive worry about not sleeping • Idiopathic Insomnia • Longstanding and persistent • Often starts in childhood • No sustained remission Chronic Insomnia

• Paradoxical Insomnia • Sleep state misperception • Report of very little to no sleep • Appear to have normal sleep on objective measures of sleep (PSG) • Evidence of altered sleep/wake system • Inadequate Sleep • Variable sleep schedule • Daytime napping • Use of sleep-disruptive products • Caffeine, tobacco • Electronic devices Chronic Insomnia

• Behavioral insomnia of childhood • Improper sleep training or limit setting • Sleep-Onset Association Type • Dependence on specific stimulation or object to fall asleep • Limit-Setting Type • stalling • Bedtime refusal • Poor limit setting by caregiver • Mixed Type • Combination of both Secondary Insomnias

• Insomnia due to a mental health disorder • Insomnia due to a medical condition • Insomnia due to a or substance Insomnia Summary

• Behavioral interventions are most • Refer if: effective • Acute Insomnia persists for unclear • Acute insomnia often resolves when reasons primary reason is resolved • Over the counter medications to help with sleep are being used • Chronic Insomnia can be nightly • Lifelong • Medications being used for • Formed by habit insomnia are not effective • Due to other medical or behavioral • Concern about underlying reason factors • OSA • Worsened by other sleep disorders • Hypoxemia • RLS • PLMD • Seizures Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Hypersomnia

• Too Much Sleep • Excessive duration of sleep • Excessive depth of sleep • Excessive frequency of sleep episodes • Risk Factors • Intrinsic • Genetic • Medication Hypersomnia

• Narcolepsy Type I • Due to loss of orexin secreting • • Sudden loss of muscle tone • Genetic predisposition • Precipitated by strong • Autoimmune component • Retain • Symptoms: • Narcolepsy Type II • Sleep attacks • No cataplexy • latency of < 8 minutes • Sleep onset REM period • Sleep Onset • Sleep/wake instability • Too sleepy in the day • Hard to sleep soundly during the night Hypersomnia

• Klein-Levin Syndrome • Hypersomnia • 2-5 week duration • Recurrent at least < every 18 months • At least one during episode: • Cognitive dysfunction • Anorexia or hyperphagia • Disinhibition • Altered Hypersomnia

• Hypersomnia due to a • Brain tumors medical disorder • CNS /lesions • Parkinson’s disease • Endocrine disorders • Post traumatic • • Genetic disorders • Metabolic • Prader Willi • Residual sleepiness in those with adequately • Moebius syndrome treated OSA • Fragile X syndrome Hypersomnia

• Hypersomnia due to a medication • Sedating medication • • Stimulant withdrawal • Hypersomnia associated with a psychiatric disorder • • Somatoform disorders • • Personality disorders • Insufficient sleep syndrome • Common in teens Hypersomnia Summary

• A known factor in • Refer if: many • Concern for underlying neurodevelopmental cause due to : • OSA • Intrinsic factors • Seizures • Medical morbidities • Overmedicated • Medications • Other sleep disorders Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Parasomnias

• Unwanted nocturnal behaviors • Simple or Complex • Routine behaviors • Inappropriate behaviors • Consciously unaware • Familial Pattern • Neither parent affected • 22% of children sleepwalk • One parent affected • 45% • Both parents affected • 60% • Predisposing, priming and precipitating factors involved NREM Parasomnias

• Often in the first third of the night • More Common in children • Typically from NREM 3 sleep • Increased with: • • Sickness • • Side effects to medications Parasomnias During NREM Sleep

•Night Terrors •Episodes of abrupt terror •Intense •Autonomic arousal •Inconsolable •Eyes open •Brief to 30+ minutes

•Confusional •Mental or confused behavior •Absence of terror or ambulation Parasomnias During NREM Sleep

• Sleep walking • Somnambulism • Sleep talking • Somniloquy • Groaning during Sleep • • Bedwetting • Enuresis • Teeth Grinding • •Sleep related eating disorders •Eating while asleep •Variant of Parasomnias During REM Sleep

•REM Behavioral Disorder • enactment •Usually Brief •Recall intact upon awaking •Themes of being pursued/fear • Associated with neurodegenerative diseases •Predate onset of Alpha- by years •Parkinson’s Disease •Multiple systems atrophy • with Lewy Bodies •Lesions affecting brain stem • •Narcolepsy •Stroke •Medications •Antidepressants Parasomnias During REM Sleep

•Recall intact upon awaking •Second half of the night

•Awake but unable to move

•Sleep related hallucinations •Hypnogogic •When falling asleep • •When waking up Summary:

• Parasomnias can be • Refer if: caused by: • Occur nightly • Sleep deprivation • Causing safety • Sickness concerns • Stress • Persist over long periods • Side effects to medications • Sleep disorders Common Sleep Problems in Developmentally Delayed Individuals • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Insomnia • Hypersomnia • Parasomnias • Sleep Related Movement Disorders Sleep Related Movement Disorders

• Restless Leg Syndrome U- Urge to move limbs R- Rest makes it worse G- Getting up/moving helps E- Evening or night S- Symptoms not due to other cause Sleep Related Movement Disorders

• Restless leg syndrome • Bothersome while awake • Associated with • Ferritin < 50 • Familial • Overlap with Periodic Limb Movements Disorder • 80% of those with RLS have PLMD Sleep Related Movement Disorders

• Periodic Limb • During Sleep • Movements interrupt/worsen sleep • Overlap with RLS • 20% of those with PLMD have RLS Sleep Related Movement Disorders

• Sleep Related Rhythmic • A “problem” when: Movement Disorder • Interferes with sleep • Repetitive, stereotyped • Daytime impairment and rhythmic motor behaviors • Risk of Self Injury • Body rocking • Head banging • Leg banging • Occurs when drowsy or asleep Sleep Related Movement Disorder Summary

• RLS can mimic insomnia • Refer if: • Check ferritin level is > 50 • Daytime functional impairment • Periodic Limb Movements may • Safety concerns not be a disorder: • if not interrupting sleep • not causing daytime dysfunction • Rhythmic Movements can be a coping tool • Dream enactment may be a harbinger of a neurodegenerative illness Developmentally Delayed Populations at High Risk for Sleep Disorders • • Myotonic Dystrophy • Obstructive Sleep • Obstructive Sleep Apnea Apnea- incidence of 50- 100% • Central Sleep Apnea • Insomnia • Central Hypersomnia • Prader Willi • Smith Magenis Syndrome • Obstructive Sleep Apnea • Circadian Rhythm • Central Hypersomnia Disorders • Nocturnal Eating • Insomnia • Obstructive Sleep Apnea Developmentally Delayed Populations at High Risk for Sleep Disorders • Syndromes with • Individuals with Dysmorphic Faces Blindness • Obstructive Sleep Apnea • Circadian Rhythm Disorders • Spectrum • Syndromes with • Insomnia • Obstructive sleep apnea • Sleep related rhythmic movement disorders • Hypersomnia • Parasomnias Case: Mr. Willi

Mr. Prader-Willi is a 39 year old morbidly obese male who reports that he wakes every morning with a headache and feels tired during the day. He sleeps an average of 12 hours a night. He often wakes up hungry in the middle of the night and sneaks to the kitchen to get a snack, while other times he wakes up with crumbs in his though does not remember getting up to eat. His roommate complains that he is “noisy” at night, which makes Mr. Willi feel persecuted. Case: Mr. Willi: Key Points

• Some developmental disorders, like Prader-Willi, have known hypersomnia • Obesity is a risk factor for OSA • Waking unrefreshed after sufficient sleep and taking day time is a major red flag for OSA • OSA is a risk factor for parasomnias due to interruptions in sleep • OSA contributes to problems with glucose regulation and impulse control • Snoring may bother others more than the patient • Obesity increases the likelihood of obesity • Morning headache due to accumulation of CO2 due to insufficient expiration/hypoventilation • Resolves when CO2 is “blown off” with normal respirations when awake Case: Ms. Nellie

Mrs. Nellie is a 57 year old female with disorder who has always had insomnia and been a nervous person, but over the last several years has been having even more difficulty falling asleep and has been waking up in the middle of the night “in a panic”. She sometimes wakes and can’t move her body for several minutes, which is very frightening to her. Now she going to sleep. She returns for follow up after starting 1mg at bedtime noting that she is falling asleep faster, but still wakes with anxiety. She has been having memory problems. She wakes to urinate at 3am and can’t return to sleep. Sometimes rocking her body helps her go to sleep. Case: Ms. Nellie Key Points • Rates of sleep apnea in women increase after • Repeated episodes of hypoxemia and increased sympathetic response can contribute to anxiety • Sedating medications decrease muscle tone in airway and can worsen OSA • Repeated hypoxemia and sleep fragmentation contributes to short term memory problems and word finding difficulties • OSA is typically worse during REM sleep • OSA increases/causes nocturia • Rhythmic movements of sleep can be a self soothing tool for some • Warrants treatment/is a disorder if causing harm or poor sleep Case: Ms. Oxy

Ms. Oxy is a 28 year old female treated with opioid pain medications for a that causes intense . She has had a few “scary” episodes where her roommate wakes and thinks she is dead because she can’t see Ms. Oxy breathing. Ms. Oxy notes sometimes waking with a racing heart. She requests something to help with her anxiety at night. Case: Ms. Roxy Key Points

• Brain injuries or malformations can the sleep wake circuitry and breathing centers in the • Opioid medications decrease respiratory drive during sleep • Brain forgets to send a signal to lungs to breathe • Decreased capacity to arouse when hypoxemic • Concurrent use of benzodiazapines and narcotic medications are a “double whammy” • Increased OSA risk from loss of muscle tone • Central sleep apnea from blocking mu receptors Case: Mr. Stumper

Mr. Stumper is a 43 year old male with trisomy 21, hypertension and diabetes, presenting for follow up. He recently started a third antihypertensive agent and increased his long acting insulin. His and diabetes are still poorly controlled. He has had difficulties with medication compliance in the past, and you suspect that he is not taking his medications as directed. When asked about this, he becomes very upset an leaves the office, tearful when noting that he is always the bad guy. Case: Mr. Stumper Key Points

• OSA increases risk of hypertension four fold • OSA prevents nocturnal dipping • Blood pressure usually drops 10-20% during sleep • OSA causes due to stress response • Also true in non-diabetics

Harding SM, J Clin Sleep Med, 2014 Case: Annie Antsy

Annie Antsy is a non-verbal 13 year old female who has recently been having agitation at bed time. She recently started menstruating She has always been a “picky” eater She was started on a to help her sleep, which seemed to make things even worse Case: Annie Antsy Key Points

• Restless legs can manifest as behavioral issues, especially in those not able to communicate • Iron deficiency is a cause of restless legs • Medications, especially antidepressants can cause restless legs • 30% of people on mirtazapine get RLS symptoms What questions do you have?

• Shanna Diaz D.O. • [email protected] • 505-272-0110

Special thanks to Frank Ralls, MD, program director of UNMH Fellowship, for sharing slide content References: • Tkacova R., McNicholas W. T., Javorsky M., et al. Nocturnal intermittent predicts prevalent hypertension in the European Sleep Apnoea Database . European Respiratory Journal. 2014;44(4):931–941. doi: 10.1183/09031936.00225113 • Muniesa M., Sánchez-De-La-Torre M., Huerva V., Lumbierres M., Barbé F. Floppy eyelid syndrome as an indicator of the presence of glaucoma in patients with obstructive sleep apnea. Journal of Glaucoma. 2014;23(1):e81–e85. doi: 10.1097/IJG.0b013e31829da19f. • Gopal, Manish et al. Investigating the Associations Between Nocturia and Sleep Disorders in Perimenopausal Women The Journal of , Volume 180 , Issue 5 , 2063 - 2067 • Ci SP1, Gao Y, Zhang XL, Mao JH, Zhao NZ, Ni JQ, Shen X, Ding M, Xu XX. Twenty years follow-up: the correlation between sleep apnea syndrome and ]. Zhonghua Jie He He Hu Xi Za Zhi. 2011 Jan ;34(1):13-6. • Harding SM. Resistant hypertension and untreated severe sleep apnea: slowly gaining insight. J Clin Sleep Med 2014;10(8):845- 846. • Walia HK, Li H, Rueschman M, et al., authors. Association of severe obstructive sleep apnea and elevated blood pressure despite anti-hypertension medication use. J Clin Sleep Med. 2014;10:835–43 • Marin JM, Carrizo SJ, Vicente E, Agusti AG. Long-term cardiovascular outcomes in men with obstructive sleep apnoea-hypopnoea with or without treatment with continuous : an observational study. Lancet. 2005;365:1046–53. • Arzt M, Young T, Finn L, Skatrud JB, Bradley TD. Association of sleep-disordered breathing and the occurrence of stroke. Am J Respir Crit Care Med. 2005;172:1447–1451 • Yaggi HK, Concato J, Kernan WN, Lichtman JH, Brass LM, Mohsenin V.. Obstructive sleep apnea as a risk factor for stroke and death. N Engl J Med (2005) 353:2034–41.10.1056/NEJMoa043104 • Munoz R, Durán-Cantolla J, Martínez-Vila E, Gallego J, Rubio R, Aizpuru F, et al. Severe sleep apnea and risk of ischemic stroke in the elderly. Stroke. 2006. September;37(9):2317–21. • Grimm W., Sass J., Cassel W., Hildebrandt O., Apelt S., Nell C., Koehler U. Severe central sleep apnea is associated with in patients with left ventricular systolic dysfunction. Pacing Clin. Electrophysiol. [(accessed on 5 September 2014)]. doi:10.1111/pace.12495. • Kauta SR, Keenan BT, Goldberg L, Schwab RJ. Diagnosis and treatment of sleep disordered breathing in hospitalized cardiac patients: a reduction in 30-day hospital readmission rates. J Clin Sleep Med. 2014;10:1051–9. • See comment in PubMed Commons belowVizzardi E1, Curnis A, Latini MG, Salghetti F, et al. Risk factors for atrial fibrillation recurrence: a literature review. J Cardiovasc Med (Hagerstown). 2014 Mar;15(3):235-53. doi: 10.2459/JCM.0b013e328358554b. • Gilat H, Shpitzer T, Guttman D, et al. Obstructive sleep apnea after radial forearm free flap reconstruction of the oral tongue. Laryngoscope. 2013;12:3223–3226.